Lee Seung Heon, Lee Sang Min, Yang Seok Chul, Yoo Chul Gyu, Kim Young Whan, Shim Young Soo, Han Sung Koo
Department of Internal Medicine, Inje University College of Medicine, Busan, Korea.
Korean J Intern Med. 2008 Dec;23(4):219-22. doi: 10.3904/kjim.2008.23.4.219.
Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.
古德综合征极为罕见。这种成人发病的病症特征为伴有免疫缺陷的胸腺瘤、B细胞和T细胞计数偏低以及低丙种球蛋白血症。最初的临床表现为胸腺瘤肿块或反复感染。古德综合征患者极易感染；常见的呼吸道感染和机会性感染可能危及生命。尽管在常见可变免疫缺陷患者中观察到了肉芽肿性肺病（古德综合征是其中一个亚型），但尚无古德综合征患者出现肉芽肿性肺病的报道。我们描述了一名53岁的男性胸腺瘤患者，他因肉芽肿性肺病和机会性感染出现呼吸道症状。尽管反复静脉注射免疫球蛋白并给予支持治疗，他仍死于无法控制的真菌感染。临床医生在患有严重反复感染尤其是机会性感染的胸腺瘤患者中应寻找免疫功能障碍的证据。
